• Annals of Clinical Neurophysiology
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• 제8권1호
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• pp.16-22
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• 2006

Sporadic inclusion body myositis (s-IBM) is an aquired slowly progressive inflammatory myopathy with unknown etiology. Although light microscopic abnormalities and characteristic histopathology on muscle biopsy distinguishes from other inflammatory myopathies, vacuolated muscle fibers, intracellular amyloid deposits or tubulofilaments in electromicroscopic findings are not definite in some patients. This review shows the prominently involved muscles in s-IBM and specific or nonspecific electrophysiologic manifestations from reported data for helping the diagnosis of definite-or probable-IBM patients. In lower limbs, the quadriceps is predominantly involved, as is iliopsoas, and tibialis anterior is common. In the upper limbs, the greatest weakness is in forearm finger flexors. Finger extensors, biceps and triceps also are moderately to prominently involved. The majority of patients demonstrate polyphasic MUAPs that are short in duration. An additional striking feature is the concomitant documentation of long-duration, large-amplitude, polyphasic MUAPs. In spite of the frequent mixed myopathic-neurogenic electromyographic findings of IBM, just like that of chronic myositis, asymmetric, slowly progressive weakness of flexor digitorum profundus or quadriceps femoris muscles after age of 50 is very necessary condition for the diagnosis of IBM.

• Annals of Clinical Neurophysiology
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• 제25권2호
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• pp.106-109
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• 2023

Inclusion body myositis (IBM) is a late-onset myopathy that manifests as distinct muscle weakness in the quadriceps, finger flexors, and ankle dorsiflexors. T-cell large granular lymphocyte (T-LGL) leukemia is a late-onset clonal disorder of CD8+ cytotoxic T-cells that is often accompanied by autoimmune diseases. To date, the association between IBM and T-LGL leukemia has been infrequently reported. Here, we report a case of a patient with T-LGL leukemia who developed IBM, along with in-depth laboratory, electrophysiological, and pathologic findings.

• Annals of Clinical Neurophysiology
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• 제4권1호
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• pp.1-6
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• 2002

The inflammatory myopathies are divided into three major and distinct subsets as polymyositis(PM), dermatomyositis(DM), and inclusion body myositis(IBM). This distinction is based on unique clinical, demographic, laboratory, histologic, therapeutic, prognostic, and immunopathologic criteria. Although the causes of PM, DM, and IBM are unknown, autoimmune mechanisms are implicated, as supported by their association with other putative or definite autoimmune diseases or viruses, the evidence for a T cell-mediated myocytotoxicity or complement-mediated microangiopathy, the presence of various autoantibodies and their response to immunotherapies. But in IBM the immune-mediated process is weaker and IBM patients do not readily respond to immunotherapies, there are convincing immunopathological signs to suggest that a definite autoimmune component, similar to that seen in PM, also plays a role in the cause of IBM.

• Pediatric Infection and Vaccine
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• 제31권1호
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• pp.136-139
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• 2024

횡문근융해증(rhabdomyolysis) 근육세포의 파괴에 따라 근세포 내 물질이 세포 외부액과 혈액으로 방출되어 증상이 나타나며, 주로 외상, 근효소 결핍, 감염, 전해질 불균형, 약물, 내분비 질환 등에 의해 유발될 수 있다. 본 연구에서는 SARS-CoV-2 감염이 확인된 7세 남아에서 나타난 횡문근융해증 사례를 보고하고자 한다. 또한, 질병 스펙트럼, 치료 및 결과를 확인하기 위한 체계적인 문헌 고찰을 수행하였다. 검색 결과, 코로나19 감염 후 7건의 횡문근융해증 보고 사례를 확인하였다. 그 중 5건은 발열이 있었으며 크레아틴 키나제(creatine phosphokinase, CK)는 3,717에서 274,664 IU/L 범위에 속하였다. 두 명은 중환자실에서 치료를 받았으며 두 명은 신장 대체 요법을 받았으며 한 명을 제외하고 모두 생존하였다. 코로나19 감염 후 횡문근융해증이 나타날 수 있으며, 근육 통증을 호소하는 소아에서의 소변 색상의 철저한 검사 및 혈액 검사를 통한 근육 효소의 평가가 진단과 치료에 도움이 될 수 있다.